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Surgical Outcomes following Resection for Sporadic Abdominal Wall Fibromatosis

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Fibromatosis can be classified according to site of origin, namely, extraabdominal, abdominal wall, or intraabdominal. This study reports on the surgical management and long-term outcomes from a single center in the management of sporadic abdominal wall fibromatosis.

Methods

Patients who underwent surgery for abdominal wall fibromatosis between 1998 and 2013 were identified from a prospectively maintained database. A retrospective review of patient demographics, tumor characteristics, surgical outcomes, operative management, and recurrence rates was performed.

Results

Fifty patients underwent resection of a primary sporadic abdominal wall fibromatosis; 48 were female, of whom 43 reported previous pregnancy. Twenty-seven patients (54 %) had prior abdominal surgery for other pathologies. Macroscopic clearance was achieved in all cases. The median size of tumors resected was 8 cm (range 3–15 cm). The abdominal wall defect was reconstructed with prosthetic mesh in 47 of 50 cases. No major postoperative complication was encountered. Microscopic margins were reported as clear (R0) in 21 of 50 cases. With a median follow-up of 6 years (range 1–15 years), 46 of 50 patients remain disease free, with a median disease-free survival of 5 years. Of these 46 disease-free patients, 13 had further pregnancies without complications from either the abdominal mesh repair or tumor recurrence.

Conclusions

For asymptomatic sporadic abdominal wall fibromatosis, observation is an accepted first-line strategy. However, in contrast to extraabdominal fibromatosis, the preferred definitive treatment is surgical resection, which is recommended as first-line therapy in symptomatic patients, selected cases when tumors are progressing, and those with tumors >7 cm.

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References

  1. Rampone B, Pedrazzani C, Marrelli D, Pinto E, Roviello F. Updates on abdominal desmoid tumors. World J Gastroenterol. 2007;13:5985–8.

    Article  PubMed  Google Scholar 

  2. Phillips SR, A’Hern R, Thomas JM. Aggressive fibromatosis of the abdominal wall, limbs and limb girdles. Br J Surg. 2004;91:1624–9.

    Article  CAS  PubMed  Google Scholar 

  3. Berri RN, Baumann DP, Madewell JE, Lazar A, Pollock RE. Desmoid tumor: current multidisciplinary approaches. Ann Plast Surg. 2011;67:551–64.

    Article  CAS  PubMed  Google Scholar 

  4. Colombo C, Foo WC, Whiting D, et al. FAP-related desmoid tumors: a series of 44 patients evaluated in a cancer referral center. Histol Histopathol. 2012;27:641–9.

    PubMed  Google Scholar 

  5. Stoeckle E, Coindre JM, Longy M, et al. A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases. Eur J Surg Oncol. 2009;35:129–34.

    Article  CAS  PubMed  Google Scholar 

  6. Bertani E, Chiappa A, Testori A, et al. Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature. Ann Surg Oncol. 2009;16:1642–9.

    Article  PubMed  Google Scholar 

  7. Li M, Cordon-Cardo C, Gerald WL, Rosai J. Desmoid fibromatosis is a clonal process. Hum Pathol. 1996;27:939–43.

    Article  CAS  PubMed  Google Scholar 

  8. Sakorafas GH, Nissotakis C, Peros G. Abdominal desmoid tumors. Surg Oncol. 2007;16:131–42.

    Article  PubMed  Google Scholar 

  9. Sturt NJ, Clark SK. Current ideas in desmoid tumours. Fam Cancer. 2006;5:275–85; discussion 287–8.

  10. Kasper B, Strobel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16:682–93.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  11. Colombo C, Miceli R, Lazar AJ, et al. CTNNB1 45F mutation is a molecular prognosticator of increased postoperative primary desmoid tumor recurrence: an independent, multicenter validation study. Cancer. 2013;119:3696–702.

    CAS  PubMed  Google Scholar 

  12. Barham W, Frump AL, Sherrill TP, et al. Targeting the wnt pathway in synovial sarcoma models. Cancer Discov. 2013;3:1286–301.

    Article  CAS  PubMed  Google Scholar 

  13. Polakis P. Wnt signaling and cancer. Genes Dev. 2000;14:1837–51.

    CAS  Google Scholar 

  14. Bonvalot S, Ternes N, Fiore M, et al. Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol. 2013;20:4096–102.

    Article  PubMed  Google Scholar 

  15. Latchford AR, Sturt NJ, Neale K, Rogers PA, Phillips RK. A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis. Br J Surg. 2006;93:1258–64.

    Article  CAS  PubMed  Google Scholar 

  16. Catania G, Ruggeri L, Iuppa G, Di Stefano C, Cardi F, Iuppa A. Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery. Updates Surg. 2012;64:43–8.

    Article  PubMed  Google Scholar 

  17. Sutton RJ, Thomas JM. Desmoid tumours of the anterior abdominal wall. Eur J Surg Oncol. 1999;25:398–400.

    Article  CAS  PubMed  Google Scholar 

  18. Wilkinson MJ, Fitzgerald JE, Thomas JM, Hayes AJ, Strauss DC. Surgical resection for non-familial adenomatous polyposis-related intra-abdominal fibromatosis. Br J Surg. 2012;99:706–13.

    Article  CAS  PubMed  Google Scholar 

  19. Awwad J, Hammoud N, Farra C, Fares F, Abi Saad G, Ghazeeri G. Abdominal wall desmoid during pregnancy: diagnostic challenges. Case Rep Obstet Gynecol. 2013;2013:350894.

    PubMed Central  PubMed  Google Scholar 

  20. Salas S, Dufresne A, Bui B, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol. 2011;29:3553–8.

    Article  PubMed  Google Scholar 

  21. Bonvalot S, Desai A, Coppola S, et al. The treatment of desmoid tumors: a stepwise clinical approach. Ann Oncol. 2012;23(Suppl. 10):x158–66.

    Article  PubMed  Google Scholar 

  22. Gronchi A, Colombo C, Le Pechoux C, et al. Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm—a position paper from the Italian and the French Sarcoma Group. Ann Oncol. Dec 9 2013 (epub ahead of print).

  23. Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587–93.

    Article  PubMed  Google Scholar 

  24. Domont J, Salas S, Lacroix L, et al. High frequency of beta-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management. Br J Cancer. 2010;102:1032–6.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  25. Lazar AJ, Tuvin D, Hajibashi S, et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol. 2008;173:1518–27.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  26. Peng PD, Hyder O, Mavros MN, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19:4036–42.

    Article  PubMed Central  PubMed  Google Scholar 

  27. Johner A, Tiwari P, Zetler P, Wiseman SM. Abdominal wall desmoid tumors associated with pregnancy: current concepts. Expert Rev Anticancer Ther. 2009;9:1675–82.

    Article  PubMed  Google Scholar 

  28. De Cian F, Delay E, Rudigoz RC, Ranchere D, Rivoire M. Desmoid tumor arising in a cesarean section scar during pregnancy: monitoring and management. Gynecol Oncol. 1999;75:145–8.

    Article  PubMed  Google Scholar 

  29. Gronchi A, Raut CP. Optimal approach to sporadic desmoid tumors: from radical surgery to observation. Time for a consensus? Ann Surg Oncol. 2012;19:3995–7.

    Article  PubMed  Google Scholar 

  30. Mullen JT, Delaney TF, Kobayashi WK, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol. 2012;19:4028–35.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Academic Surgery, Sarcoma and Melanoma Unit, The Royal Marsden NHS Foundation Trust, London, UK

    Michelle J. Wilkinson MRCS, Katie E. Chan MRCS, Andrew J. Hayes FRCS, PhD & Dirk C. Strauss FRCS

Authors
  1. Michelle J. Wilkinson MRCS
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  2. Katie E. Chan MRCS
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  3. Andrew J. Hayes FRCS, PhD
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  4. Dirk C. Strauss FRCS
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Correspondence to Dirk C. Strauss FRCS.

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Wilkinson, M.J., Chan, K.E., Hayes, A.J. et al. Surgical Outcomes following Resection for Sporadic Abdominal Wall Fibromatosis. Ann Surg Oncol 21, 2144–2149 (2014). https://doi.org/10.1245/s10434-014-3618-5

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  • DOI: https://doi.org/10.1245/s10434-014-3618-5

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